CJD Deaths Rising--Mad Cow Disease May Kill 500,000 Britons

Subject: Deaths Tied to Mad Cow Disease on the Rise
New York Times, Tuesday, July 25, 2000
By Sandra Blakeslee

Deaths from the human form of mad cow disease appear to be increasing,
British health officials have reported, but they say it is still unclear
whether the increase is the start of an epidemic or merely a statistical
blip.

So far this year, 14 Britons have died of the disease. That is as many
as died all last year, and five others are known to be dying from the
disease, which is always fatal.

If the trend continues and an epidemic is in its early stages, experts
estimate that as many as 500,000 Britons could die over the next 30
years from the disease, which is contracted by eating infected beef
products.

Even if the numbers begin to fall or hold steady, they said, hundreds or
thousands of people are going to die from the disease, called variant
Creutzfeldt-Jakob Disease, which literally eats holes in the brains of
its victims.

"I am worried about this year's figures," Dr. Roy Anderson, a zoologist
from Oxford University who has studied the epidemic, told The
Independent, a British newspaper, last week.

Dr. Anderson said Britain was just now seeing the consequences of
exposure to the disease in cows in the early 1980's. He said in humans
the disease had a "long incubation period, then cases appearing in a
trickle."

The rise in deaths now fits that pattern, he said.

"That's what you expect in an epidemic." Other experts said the trend
was less certain.

"It's hard to know what the new numbers mean," Dr. Peter Smith, an
epidemiologist at the London School of Hygiene and Tropical Medicine,
said in a telephone interview. Dr. Smith is acting director of the
Spongiform Encephalopathy Advisory Council, which advises the British
government on the disease.

Dr. Smith said that the number of cases remained flat for the last four
years, and that something now appeared to have been "switched on." Since
it first appeared in humans in 1996, a total of 74 people in Britain, 2
in France and 1 in Ireland are dead or dying from the disease, he said.

The increase in deaths this year "is surprising, and it is of some
concern," Dr. Smith said.

"But it does not necessarily portend a large epidemic."

The Health Department issues a monthly bulletin on the number of cases,
Dr. Smith said. The next bulletin is due on Aug. 7.

Mad cow disease first appeared in the mid-1980's when British cattle
began falling ill with a mysterious brain malady.

The epidemic was traced to protein feed supplements infected with brain
and nervous tissue extracted from sick cows.

Since then, more than 176,000 cows have died from the disease and 4
million more were destroyed to prevent the disease from further
spreading.

But it soon turned out that the infection could spread from cows to
humans through eating contaminated beef products, a fact scientifically
confirmed only last year.

The transfer to humans was first suspected in 1996, when 10 young people
died with spongy holes in their brains. Until then, such symptoms were
found only in much older people who died from a form of C.J.D. not
related to eating cattle.

But since the disease may have an incubation period of more than 25
years, the question remains how many Britons may be infected and will
eventually die from it? Millions of people probably came into contact
with infected meat, though it is not clear how many will actually
contract the disease, Dr. Smith said.

So far all those infected have possessed a particular genetic trait that
apparently predisposed them to the disease. At least 40 percent of the
British population shares that trait, which involves a variation of the
prion protein, according to experts.

Health officials announced last week that they had identified a probable
cluster of cases in Queniborough, a small village about 100 miles north
of London. Four young adults from the area have died, apparently from
the disease, in the last two years and fifth person, who just turned 25,
is near death.

Epidemiologists are combing the village for clues to what the victims
had in common and to help them better understand how the disease spreads
from cows to people.

Researchers are handing out questionnaires to the village's 2,297
residents asking them what they ate 10 and 15 years ago. They are also
investigating 10 local slaughterhouses where cattle parts, including
offal, were often turned into specialty meats.

It is possible that a locally slaughtered "mad" cow made its way into
sausages eaten by Queniborough's children, said Dr. Philip Monk, who is
an expert in communicable diseases at the Leicestershire Health Authority.

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